肺血栓塞栓症を合併した不整脈源性右室心筋症の1剖検例

Arrhythmogenic Right Ventricular Cardiomyopathy with Pulmonary Thromboembolism:An autopsy report

要旨　患者は60歳，男性．以前より高血圧症および心電図異常(wide QRS波，イプシロン波)を指摘されていた．入院の10日前より食欲不振，動悸，全身倦怠感を自覚しており，胸痛，呼吸困難にてショック状態で入院となった．肺動脈主幹部の血栓や深部静脈血栓症の存在は明らかではなかったが，著明な右心系拡大と，心エコー観察中に血栓が右室から肺動脈へ飛散する像を認め急性肺血栓塞栓症と診断した．ただし，全身状態が悪くその後死亡した．剖検にて右室は心基部から心尖部にかけて広範囲に脂肪組織に置換されており，診断基準と合わせて不整脈源性右室心筋症と診断した．右室内(三尖弁直下，肉柱間，心尖部)には小豆大の血栓を多数認め，固定後の肺動脈内には比較的新しい血栓を多数認めた．不整脈源性右室心筋症で右室由来の血栓による急性肺血栓塞栓症を来した剖検症例を経験したので報告する．

　A 60-year-old man was pointed out to have hypertension and ECG abnormality(wide QRS wave and ε wave). He had noticed appetite loss, palpitation, and general fatigue 10 days before his admission. He felt chest pain and dyspnea, and came to our hospital in a vital state but in shock. Apparent thrombus in the pulmonary truncus and deep vein thrombosis was ruled out, but enlargement of the right atrium and ventricle was observed. Scattering of the thrombus from the right ventricle to the pulmonary artery was observed during ultrasound cardiography, and he was diagnosed as having acute pulmonary thromboembolism. In the autopsy, it was found that his right ventriclar muscle had been replaced by fatty tissue from base to apex of the right ventricle extensively, and the diagnosis was arrhythmogenic right ventricular cadiomyopathy(ARVC)when referred to the criteria. Many small beans-size thrombi were observed in the right ventricle, and many fresh thrombi were also observed in the pulmonary arteries after fixation of the lung tissue. The final diagnosis after the autopsy was ARVC complicated by pulmonary thromboembolism, and the thrombus was considered to have originated from the right ventricle.